Acute pandysautonomia

Dr. Roos of the University of Chicago Medical Center serves on the Scientific Advisory Board of Revalesio Corporation and a Data and Safety Monitoring Board for a clinical trial of a Mallinckrodt Pharmaceuticals product, has received consulting fees from M-T Pharmacy and Best Doctors and holds shares in Amgen, Merck, Ionis, and GE.

)Originally released May 8, 1995; last updated September 28, 2017; expires September 28, 2020

The syndrome of acute pandysautonomia is characterized by acute onset (onset to peak symptoms within 1 month) of severe and disabling autonomic failure affecting sympathetic, parasympathetic, and enteric functions. If initial symptoms are episodic (syncope) the onset may not be as precise, and for the purpose of this article subacute (1 month to 2 months) presentations are also included. There may be an inciting event that accounts for unrestrained immune activation, such as an acute infection, vaccination, surgery, or an occult cancer. Acute metabolic and toxic exposure can also cause similar manifestations. The clinical features consist of variable involvement of sympathetic, parasympathetic, and enteric portions of the autonomic nervous system. Other portions of the nervous system may be involved less frequently: brain, somatic sensory, and rarer motor nerve fibers. Symptomatic treatment is complex and is helpful in supporting function. If an autoimmune etiology is identified immunomodulatory treatments should be employed early in the course to avoid progressive disability. In this article, the author discusses various clinical presentations with emphasis on early diagnosis, current pathophysiology, and treatment.

• In addition to symptomatic and supportive treatment, early immunomodulating therapies should be considered in order to avoid long-term disability.

Historical note and terminology

The syndrome of acute pandysautonomia was first reported in the neurologic literature by Young and colleagues in 1969, and was later discussed in more detail in the same patient as "pure pandysautonomia with recovery" (Young et al 1975). The patient showed "severe postural hypotension without change in heart rate, leading occasionally to fainting without change in heart rate, total absence of sweating, extreme dryness of eyes, nasal and oral mucous membranes, midposition nonreactive pupils, absence of bowel sounds and a hypotonic weak bladder." Appenzeller and Kornfeld first coined the term "acute pandysautonomia" (Appenzeller and Kornfeld 1973), and similar cases were also reported later as acute autonomic neuropathy, acute panautonomic neuropathy, acute idiopathic pandysautonomia, and idiopathic autonomic neuropathy (Hopkins et al 1974; Low et al 1983; Suarez et al 1994).

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